Article
An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes.
Human molecular genetics - 1 Sept 2016
Southwell Amber L, Smith-Dijak Amy, Kay Chris, Sepers Marja, Villanueva Erika B, Parsons Matthew P, Xie Yuanyun, Anderson Lisa, Felczak Boguslaw, Waltl Sabine, Ko Seunghyun, Cheung Daphne, Dal Cengio Louisa, Slama Ramy, Petoukhov Eugenia, Raymond Lynn A, Hayden Michael R
Abstract excerpt
Huntington disease (HD) model mice with heterozygous knock-in (KI) of an expanded CAG tract in exon 1 of the mouse huntingtin (Htt) gene homolog genetically recapitulate the mutation that causes HD, and might be favoured for preclinical studies. However, historically these mice have failed to phenotypically recapitulate the human disease. Thus, homozygous KI mice, which lack wildtype Htt, and are much less...
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